Central Pontine Myelinolysis
Central Pontine Myelinolysis
Central Pontine Myelinolysis
Abstract
Central Pontine Myelinolysis (CPM) commonly presents as a complication of treatment in patients
with profound life threatening hyponatraemia. It occurs when the sodium level is corrected too rap-
idly. Hyponatraemia should never be corrected at a rate greater than 8-10mmol/L of sodium per
day. Rapid correction causes extracellular tonicity and will continue to drive water out of the brain’s
cells leading to cellular dysfunction.
Frequent clinical signs include dysphagia, dysarthria, diplopia and acute para/quadraparesis.
Patients can also experience locked in syndrome, where cognitive function is intact but all muscles
are paralysed with the exception of eye blinking.
CPM gets its name as it occurs when cell dysfunction causes destruction of the myelin sheath of
nerve cells in the brain stem, more specifically the pons. It is associated with poor prognosis and
prevention is of primary importance.
Freddy is a 35 year old freelance graphic designer, fitness instructor and ultra-marathon runner. In
October 2011 he competed in the Sahara Marathon in Morocco, a 6 day 255km ultra-marathon. At
the end of the third day Freddy was found collapsed and vomiting. He was confused and was suf-
fering severe leg cramps. The next morning Freddy suffered a single convulsive episode and was
subsequently transferred to a hospital in Egypt.
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Australasian Journal of Neuroscience Volume 25 Ɣ Number 1 Ɣ May 2015
has occurred. The most commonly ob- sis has led to better prognosis for many.
served symptoms of CPM are: Most individuals improve gradually but con-
- acute quadraparesis tinue to live with significant deficits.
- dysphagia
- dysarthria Case Study
- diplopia Freddy (not his real name) is a 35 year old
- loss of consciousness freelance Graphic Designer and a fitness
enthusiast. In his spare time he is a fitness
The key features of the neurological exam instructor and ultra-marathon runner. In
include confusion, horizontal gaze paralysis 2011 Freddy went to Morocco to compete in
and spastic quadriplegia. Increased limb the Marathon des Sables or the Marathon of
tone, weakness, hyperactive reflexes and the Sands which is a 6 day stage race which
Babinski sign are all typical of lesions involv- covers 250 km through the Sahara Desert.
ing upper motor neurons. This multiday event is held every year and is
considered the toughest foot race on Earth.
In some instances, brain damage to the
pons from rapid myelinolysis of the corti- On day 3 of the event Freddy was acting
cobulbar and corticospinal tracts in the normally and interacting with friends. Late in
brainstem leading to “Locked in Syndrome” the evening Freddy was found near his tent
or death. “Locked in Syndrome” is when an suffering leg cramps, he was confused and
individual has full consciousness and cogni- it was evident that he had been vomiting. He
tive function intact, but has severe paralysis was assessed by race doctors and given
of the voluntary motor system where move- fluids, and it was recommended that he be
ment and communication is not possible, transferred to the closest major hospital.
however patients are able to blink or move
their eyes vertically. Delerium and coma are Whilst travelling to the base camp the next
extremely common in CPM (Hickey, 2003). day Freddy had a single convulsive episode,
This results from lesions in the pontine teg- and became more confused and agitated.
mentum and/or thalamus. Magnetic Reso- Subsequently he was rushed by ambulance
nance Imaging (MRI) is the modality of to a major hospital in Egypt. On arrival to
choice. MRI images demonstrate hyper- the hospital it was reported that two other
intense or bright areas where demyelination athletes were admitted to the same hospital
has occurred (Hromanik, 2010). with similar conditions. Freddy was admitted
on 8th October 2011, his blood profile was:
Treatment of CPM x Serum NaΆ was 108mmol/L
Once demyelination of the pons has started x Rest of electrolytes were normal
there is no cure or very little treatment that x Creatinine Kinase (CK) was 30,000u/
can be offered (Abbott, Silber, Felber & Ek- L(<1,000u/L)
po, 2005). The only treatment is that of the
symptoms alone. Elevated levels of the enzyme CK indicate
These include: muscle damage or muscle strain such as in
- physical therapy, to improve balance and the case of a heart attack or the muscles
retain range of motion in limbs. being overworked (Medical Health Tests,
- a dopagenic medication such as Levodo- 2011). In this case the excessive running in
pa to increase dopamine and control trem- the marathon done by Freddy could have
ors and the difficulties with swallowing and contributed to his high CK levels.
speech.
Freddy’s vital signs were as follows BP
Nurses can assist in the treatment, mainly 125/53, HR 92bpm, RR 25bpm, central tem-
by keeping the patient comfortable. It is im- perature was 42.9Ԩ, and Sp02 99% on RA.
perative to source an effective communica- His GCS was 13 (E4 V4 M5).
tion tool, to allow a patient to be involved in
their care and keep some “normality” in their There was nothing obvious reported on both
life. Nurses can provide family support and his CT and MRI. It was decided that treat-
refer the patient to the necessary Allied ment would start focusing on, the correction
Health teams for ongoing management. of electrolyte imbalance, treatment of rhab-
domyolysis, the management of disturbed
Prognosis level of consciousness, and the treatment of
Although CPM was considered to have the fever. It was recommended that Freddy
mortality rate of 50% or more, early diagno-
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Australasian Journal of Neuroscience Volume 25 Ɣ Number 1 Ɣ May 2015
stay in the ICU for closer monitoring. were unsure of what was happening to Fred-
dy, a reporting medical officer said... “I can-
An unknown IVF was infused at 200ml/hr and not explain his elevated enzymes...and alt-
hypertonic saline was given at a rate of 20ml/ hough they have dropped, they still remain in
hr to correct symptomatic hyponatraemia. the thousands.....I am unclear of what is go-
Freddy was administered antipyretics to con- ing on. The low Na+ and the dehydration
trol fever and he was also started on antimi- have been corrected, and there is nothing
crobial and anti-viral medication to control obvious in the brain neither on his CT or MRI.
possible infection, as well as anticonvulsants There are no signs of infection, his breathing
for his reported seizures. is normal, his chest is clear, and his abdo-
men is soft.....I am treating him conservative-
On 9th October 2011, approximately around ly without any specific diagnosis at hand.”
36 hours after treatment had started it was The medical staff decided that Freddy had
documented that Freddy’s CK levels had sig- treatment to the level of care able to be pro-
nificantly declined but were still slightly ele- vided and that it would be beneficial for him
vated and his Na+ levels had started to rise to travel to a higher level of care for re-
‘gradually’ reaching 138mmol/L. However evaluation and second opinion. A German
Freddy’s LOC did not improve, the medical hospital answered the call and agreed to ac-
staff mentioned he had become ‘locked in’, cept Freddy under their care.
with no eye fixation, increased motor tone
and spasticity, and an inability to walk or sit Freddy arrived in Germany 12th October
independently. He also had 2 or 3 general- 2011, ninety-six hours after initial onset. Pri-
ised seizures around this time. The team mary reports state that “the patient is awake
decided that if Freddy’s LOC didn’t improve but unresponsive with present mutism, the
in the next few days they would do a spinal general muscle tone of his extremities and
tap/lumbar puncture. torso are increased, his neural tension tests
A MRI of the brain (Figure 1) was performed were positive, and he showed negative Ba-
to exclude central causes, but showed that binski signs bilaterally.”
no abnormalities were detected. On October
10th 2011, Freddy’s vital signs were - BP Initial MRI showed abnormalities in the left
110/50, HR 89bpm, RR 21bpm, Sp02 99% corpus callosum, para-hippocampal gyrus
on RA, temperature 38.2°C, and urine output and left occipital lobe, his EEG was abnormal
200mls/hr. and suggestive of subcortical dysfunction,
without potentials for epilepsy, and CSF re-
sults from spinal tap/lumbar puncture showed
only a minor disturbance of the barrier func-
tion, and no indications for an inflammatory
infection of the CNS. Given these results
and the patient history the medical staff in
Germany decided to initiate anti-epileptic
therapy in the form of Levetiracetam. Freddy
tolerated this and the dose was gradually
increased to 2000mg daily with no further
seizures occurring. Freddy was also pre-
scribed a dopagenic medication to help facili-
tate activation of his muscles, and a muscle
relaxant to reduce his increased limb tone.
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Australasian Journal of Neuroscience Volume 25 Ɣ Number 1 Ɣ May 2015
Freddy spent 16 weeks in the BIU at West- www.medicalhealthtests.com/askquestion/29/
what-is-the-significance-of-a-slightly-elevated-
mead Hospital he was discharged home un-
cr.html
der the care of his parents 5th April 2012, af- Medline Plus, viewed 3rd June 2013
ter several successful home trials. On dis- http://www.nlm.nih.gov/medlineplus/ency/article
charge Freddy was able to reliably indicate National Institute of Neurological Disorders and Stroke,
yes/no using a non-verbal thumbs up or viewed18 t h August 2013,
www.ninds.nih.gov/disorders/central_pontine/
down in response to direct questions, use an central_pontine_myelinolysis.htm
increased portion of real and non-real words, Pradhan, S., Jha, R., Singh, M., Gupta,S., Phadke, R. &
and he was able to understand and follow Kher,V. (1995). Central pontine myelinolysis
following ‘slow’ correction of hyponatraemia.
simple routine spoken commands such as ‘sit Journal of Clinical Neurology and Neurosurgery,
down’. Freddy was able to use some routine 97 (4), 340-343.
gestures in a functional manner such as Stöppler, M. (2013). Hyponatremia, viewed 3rd June,
pointing to a comb and gesturing brushing his 2
http://www.medicinenet.com/hyponatremia/
hair, understand the spoken function of most page3.htm
daily objects, reliably follow conversations
with staff and family, demonstrate active lis-
tening skills such as looking at the other per-
son, nodding his head and using fillers such
as ‘yeah’ or ‘okay’.
Conclusion
Freddy now lives at home with his parents
who are his guardians. Although he will never
be able to live independently by himself re-
ports from his family are that his sense of
humour remains the same, as he constantly
is playing tricks on his family which he finds
amusing. He does have good and bad days
but his family report that he is a pleasure to
be around and most pleasing of all for Freddy
is that he has joined a running club which he
attends three times a week which he is en-
joys immensely.
References
Abbott, R., Silber, E., Felber, J., & Ekpo, E. (2005).
Osmotic demyelination syndrome, BMJ: British
Medical Journal, 331 (7520), 829 – 830.
Hromanik, K. (2010). Central pontine myelinolysis.
Journal of Emergency Nursing 36 (4), 324 – 326.
Hickey, J. (2003). The Clinical Practice of Neurological
and Neurosurgical Nursing 5 t h Ed,
Lippincott,Williams and Wilkins, Philadelphia.
Medical Health Tests, viewed 3rd June 2013 http://
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